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Prion disease: Systematic review

Systematic review of therapeutic interventions in human prion disease

What treatments have been tried in prion disease and how successful have they been?

What was this study about?

Prion diseases are very rare but fatal brain diseases. Creutzfeldt-Jakob disease (CJD) is the most common of all the human prion diseases. There are 4 different types of CJD.

  • Sporadic CJD (sCJD): this is the most common type of CJD, has no known cause and appears 'out of the blue'.
  • Genetic CJD or inherited prion disease (gCJD or iPD): runs in families.
  • Iatrogenic CJD (iCJD): is accidentally transmitted by medical or surgical procedures.
  • Variant CJD (vCJD): is transmitted through contaminated meat and blood transfusions and is also known as the human form of mad cow disease.

So far no-one has found a cure or effective treatment for CJD and other human prion diseases. Doctors have tried different treatments in many studies. Therefore, thousands of patients with prion disease have been observed or treated over the last 30 years.

Therefore, we did a systematic review. This brought together information from the reports of published studies looking at untreated patients with prion disease. This aimed to see how long they lived and what their symptoms were. We also brought together information from the reports of published studies looking at treated patients with prion disease. This aimed to see if patients who had treatment lived longer or had different symptoms compared to patients who didn't receive any treatment.

What difference did this study make?

This review is the best summary of treatment for prion disease so far. A lot of research has been done, but many studies had very few patients in them. For some studies there wasn't enough information about the patients or the treatment they had. For other studies the results were not explained very clearly. This made it hard to be sure about which treatments are best for prion disease. This means that bigger and better studies are needed, and randomised controlled trials would be best. To do these, doctors, and patients and their families across the world will have to work together.

Stewart LA, Rydzewska LHM, Keogh GF, Knight RSG. Systematic review of therapeutic interventions in human prion disease. Neurology. 2008;70(15):1272-81

 

Type of study

Meta-analyses

Contact details

MRCCTU.meta-analysis@ucl.ac.uk

Who funded the study?

The NHS Research and Development Programme and the Medical Research Council

When did it take place?

This review was published in 2008. The studies included were published between 1971 and 2005.

Where did it take place?

The review was done at the MRC Clinical Trials Unit. But it brought together the results of studies from all over the world.

Who was included?

Patients with prion disease who were studied, but were not given any treatment for their disease (patient series). Also, patients with prion disease that took part in any type of study where they were given treatment. The study brought together 140 patient series. These included over 9000 patients from 32 countries. The study also brought together 33 treatment studies. These included 149 patients.